Bassen-Kornzweig syndrome

Bassen-Kornzweig syndrome is a rare disease passed down through families in which a person is unable to fully absorb dietary fats through the intestines.

Causes of Bassen-Kornzweig syndrome

Bassen-Kornzweig syndrome is caused by a defect in a gene that tells the body to create lipoproteins (molecules of fat combined with protein). The defect makes it hard for the body to properly digest fat and essential vitamins.

Potential Complications

When to Contact a Health Professional

Call your health care provider if your infant or child has symptoms of this disease. Genetic counseling can help families understand the condition and the risks of inheriting it, and learn how to care for the patient.

Prevention of Bassen-Kornzweig syndrome

High doses of fat-soluble vitamins may slow the progression of some problems, such as retina damage and decreased vision.